61 The nailfold videocapillaroscopy in pediatric Behçet’s disease: a multi-center study

Abstract Background Behçet's disease (BD) is a chronic inflammatory disease characterized by recurrent oral aphthous and genital ulcers accompanied by eye, joint, skin, gastrointestinal and central nervous system involvement. The vascular involvement may affect both the arterial and venous systems. Nailfold videocapillaroscopy (NVC) is an easy and non-invasive method used in the evaluation of microcirculation. With this study, we aimed to find the characteristics and prevalence of nailfold capillary alterations in patients with juvenile BD and to analyze their possible relationship between clinical characteristics and activity of the disease. Methods Patients aged 5–21 years with a diagnosis of juvenile BD and followed up for at least six months were included in the study. Demographic and clinical characteristics of the patients were recorded. NVC was performed on 8 fingers of both hands, excluding the thumbs, and four consecutive non overlapping fields for each of fingers were evaluated (32 fields per patient). Capillary density, capillary width (arterial width, venous width, apical loop), capillary morphology and the presence of meandering capillary, micro hemorrhage, avascular area, neoangiogenesis, capillary ramification were evaluated from the images. Capillary morphology were evaluated by classifying them into four groups as normal, minor abnormalities, major abnormalities and scleroderma pattern. The presence of abnormilities in at least two fingers were recorded as capillary abnormality. The semi quantitative rating score 1–3 was applied for each capillaroscopic alteration. Results 37 patients from 6 pediatric rheumatology centers were included in the study. The mean age of patients was 17 years (IOR 13–19) and 20 (54.1%) of them were girls. The patients were evaluated in four clusters according to their clinical presentations. Nineteen patients had mucocutaneous involvement, 9 patients had uveitis, 8 patients had vascular and neurological involvement, and 4 patients had gastrointestinal system involvement. During the follow-up period, genital ulcers developed in 22 patients, erythema nodosum in 9 patients, pseudofolliculitis in 18 patients, uveitis in 10 patients, vascular involvement in 8 patients, and neurological involvement in 5 patients. Anterior uveitis was present in five, posterior uveitis in three, panuveitis in one, and retinal vasculitis in three of the patients with ocular involvement. Four patients had lower extremity venous thrombosis, three patients had central nervous system (CNS) thrombosis, and one patient had both lower extremity and CNS thrombosis. When capillary morphology was evaluated; normal morphology was present in 16 patients, minor abnormality in 13 patients, and major abnormality in 8 patients. Median capillary density was 8, capillary length was 325 µm, arterial width was 12 µm, venous width was 16 µm, apical loop width was 18 µm, capillary width was 39 µm, and intercapillary distance was 107 µm. Neoagiogenesis was seen in 13 patients, enlarged capillaries in 12 patients, capillary meandering in 9 patients, bushy capillaries in 5 patients, bizarre capillaries in 4 patients, and microhemorrhage in 3 patients. Neoangiogenesis was found to be significantly more common in the NVC evaluation of patients with lower haemoglobin values at the time of diagnosis (p = 0.014). Conclusion NVC is an in vivo, non-invasive, and inexpensive imaging technique that allows the direct observation of the capillary network in living tissue throughout the skin and it may be preferred in juvenile BD for evaluating microvascular involvement. 61 Figure 1 Capillaroscopic alterations of patients


Background
Behç et's disease (BD) is a chronic inflammatory disease characterized by recurrent oral aphthous and genital ulcers accompanied by eye, joint, skin, gastrointestinal and central nervous system involvement. The vascular involvement may affect both the arterial and venous systems. Nailfold videocapillaroscopy (NVC) is an easy and non-invasive method used in the evaluation of microcirculation. With this study, we aimed to find the characteristics and prevalence of nailfold capillary alterations in 61 Figure 1. Capillaroscopic alterations of patients patients with juvenile BD and to analyze their possible relationship between clinical characteristics and activity of the disease. Methods Patients aged 5-21 years with a diagnosis of juvenile BD and followed up for at least six months were included in the study. Demographic and clinical characteristics of the patients were recorded. NVC was performed on 8 fingers of both hands, excluding the thumbs, and four consecutive non overlapping fields for each of fingers were evaluated (32 fields per patient). Capillary density, capillary width (arterial width, venous width, apical loop), capillary morphology and the presence of meandering capillary, micro hemorrhage, avascular area, neoangiogenesis, capillary ramification were evaluated from the images. Capillary morphology were evaluated by classifying them into four groups as normal, minor abnormalities, major abnormalities and scleroderma pattern. The presence of abnormilities in at least two fingers were recorded as capillary abnormality. The semi quantitative rating score 1-3 was applied for each capillaroscopic alteration. Results 37 patients from 6 pediatric rheumatology centers were included in the study. The mean age of patients was 17 years  and 20 (54.1%) of them were girls. The patients were evaluated in four clusters according to their clinical presentations. Nineteen patients had mucocutaneous involvement, 9 patients had uveitis, 8 patients had vascular and neurological involvement, and 4 patients had gastrointestinal system involvement. During the follow-up period, genital ulcers developed in 22 patients, erythema nodosum in 9 patients, pseudofolliculitis in 18 patients, uveitis in 10 patients, vascular involvement in 8 patients, and neurological involvement in 5 patients. Anterior uveitis was present in five, posterior uveitis in three, panuveitis in one, and retinal vasculitis in three of the patients with ocular involvement. Four patients had lower extremity venous thrombosis, three patients had central nervous system (CNS) thrombosis, and one patient had both lower extremity and CNS thrombosis. When capillary morphology was evaluated; normal morphology was present in 16 patients, minor abnormality in 13 patients, and major abnormality in 8 patients. Median capillary density was 8, capillary length was 325 mm, arterial width was 12 mm, venous width was 16 mm, apical loop width was 18 mm, capillary width was 39 mm, and intercapillary distance was 107 mm. Neoagiogenesis was seen in 13 patients, enlarged capillaries in 12 patients, capillary meandering in 9 patients, bushy capillaries in 5 patients, bizarre capillaries in 4 patients, and microhemorrhage in 3 patients. Neoangiogenesis was found to be significantly more common in the NVC evaluation of patients with lower haemoglobin values at the time of diagnosis (p ¼ 0.014). Conclusion NVC is an in vivo, non-invasive, and inexpensive imaging technique that allows the direct observation of the capillary network in living tissue throughout the skin and it may be preferred in juvenile BD for evaluating microvascular involvement. Background Spinal pain is a frequent symptom whose aetiology can be extremely varied. It is not only the prerogative of adults; it is frequent in children and particularly in adolescents. Inflammatory spinal pain is a warning sign that should prompt further investigations to rule out an underlying pathology such as infectious spondylodiscitis, malignant pathology, ankylosing spondylitis or SAPHO syndrome. In this case, we report on a 15-year-old child with uncommon inflammatory spinal pain that caused diagnostic difficulties. Observation The child B.A., aged 15 years, with no previous history, consulted for inflammatory back pain that had been evolving for >3 months in a context of apyrexia and conservation of the general state, associated with pain of the anterior thoracic wall. The clinical examination revealed an exaggerated dorsal kyphosis, a lumbar spinal syndrome. The rest of the joint examination was without abnormalities and the somatic examination revealed a retention acne. Biologically, there was no inflammatory syndrom, brucella serology and tubeculin TST were negative. An X-ray and a CT scan of the dorsal-lumbar spine showed multiple bone erosions of the vertebral plates and a fracture of the T6 and T7 vertebrae with global disc pinching, without recession of the posterior wall or damage to the soft tissues and with exaggeration of the dorsal cyphosis at this level. The spinal MRI revealed a spondylodiscitis with hypo T1 hyper T2 signal at the T6-T7 level without any collection. An osteolytic lesion of the sternal manubrium lateralized to the left with blurred contours was objectified by the CT scan of the anterior chest wall. Bone scans revealed intense hyper fixation foci at the T6-T7 level and the sternal manubrium. MRI of the sacroiliac joints was normal. A scan-guided biopsy of the T6-T7 level did not show any specific lesion. In view of the inflammatory spinal pain, the anterior chest wall pain, the negativity of the biological tests and the imaging data, the diagnosis of incomplete SAPHO syndrome was retained after having ruled out other etiologies. SAPHO syndrome is a rare entity that refers to the association of a heterogeneous set of cutaneous and osteoarticular manifestations with a common denominator of an aseptic inflammatory process. It remains a diagnosis of elimination.

Conclusion
Spinal pain in children can be a symptom of multiple etiologies and sometimes poses a diagnostic problem. This is a particular observation and deserves to be reported because unusual clinical forms of SAPHO syndrome exist and should not be ignored.

Background
The term ''dentinogenesis imperfecta'' includes dental anomalies quite similar to hereditary opalescent dentin and osteogenesis imperfecta. We present dental findings in a boy with osteogenesis imperfecta type IV Case Presentation A young boy was referred to the pediatric department for management of vertebral compression and a recurrent femoral fracture without obvious trauma. The appearance of his teeth with the overall assessment of this patient reveals dentinogenesis imperfecta compatible with osteogenesis imperfecta type IV, associated with major vertebral involvement Discussion Osteogenesis imperfecta (OI), also known as also known as « brittle bone disease » is a heterogeneous group of rare genetic diseases involving connective tissue. A reduction in the amount of normal collagen leads to Type I, while qualitative and quantitative abnormalities in collagen synthesis lead to Types II, III and IV of OI. This type IV is subdivided into groups A and B: the group B depicting the characteristic dentinogenesis imperfecta like in our patient. Conclusion Dentinogenesis imperfecta is a major sign of osteogenesis imperfecta. Skeletal phenotype, scleral colour and deafness complete the picture allowing the final classification of this inherited disorder The peculiar bone lesions with normal dental aspects are highly suggestive of the extreme rare osteogenesis imperfecta type IV.

Background
Ectodermal dysplasias (ED) are inherited disorders involving congenital abnormalities of different ectodermal structures, the most prominent presentation being adontia/hypodontia In this article, we present a brief overview of several pediatric cases of ED with a short review of the odontological manifestations.

Case reports
We report several cases of children consulting in the pediatric department for abnormal teeth, with or without systemic manifestations.